In children with the rare disease Pfeiffer syndrome, craniofacial surgery to correct skull defects is followed by a distinct pattern of increases in body temperature, reports a study in the January Journal of Craniofacial Surgery. The journal is published by Lippincott Williams & Wilkins, a part of Wolters Kluwer Health, a leading provider of information and business intelligence for students, professionals, and institutions in medicine, nursing, allied health, pharmacy and the pharmaceutical industry.

Together with previous studies, the results suggest that the postoperative temperature spikes are normal after surgery to correct craniosynostosis even when the skull defect is the only abnormality, and not part of a larger syndrome like Pfeiffer syndrome. The study was performed by Dr. Ikkei Tamada and colleagues of the Australian Craniofacial Unit at Women’s and Children’s Hospital, Adelaide, Australia.

‘Bimodal’ Pattern Is Normal after Craniosynostosis Surgery

The researchers analyzed temperature changes after skull surgery in 21 infants and children with Pfeiffer syndrome: a rare genetic condition affecting about 1 in 100,000 people. Infants with Pfeiffer syndrome develop craniosynostosis, in which the joints (sutures) between the bones of the skull close prematurely, resulting in abnormal growth of the skull and brain. Craniofacial surgeons, in concert with other professionals, design effective treatments to correct the skull deformity, prevent complications, and promote good long-term functioning.

Patients with Pfeiffer syndrome may have other abnormalities as well, such as hearing loss and defects of the fingers and toes. This is in contrast to the more common “nonsyndromic” forms of craniosynostosis, in which the skull defect is the only abnormality.

In the children with Pfeiffer syndrome, temperature changes followed a “bimodal” pattern, with two spikes in body temperature occurring within the first 48 hours after surgery. The temperature spikes were higher and longer lasting in children with more complex procedures for example, those whose surgery took longer.

The swings in body temperature were also more pronounced, and more complicated, in children whose surgery was complicated by leakage of cerebrospinal fluid (fluid that helps to cushion the brain and spinal cord). In contrast, the temperature spikes were less prolonged in infants less than six months old.

In a previous study, Dr. Tamada and colleagues found an almost identical pattern of temperature spikes in children undergoing surgery for simple, nonsyndromic craniosynostosis. As in Pfeiffer syndrome patients, the temperature changes resolved by 48 hours after surgery, remaining normal thereafter.

The causes of the temperature changes after surgery for skull defects aren’t clear. A similar bimodal pattern has been found in patients undergoing heart surgery, but not other types of procedures.

Fever is one of the most important signs of complications after surgery, especially infection. However, the new results suggest that increases in body temperature after craniofacial surgery for craniosynostosis alone or as part of a syndrome like Pfeiffer syndrome are normal and apparently not related to infection.

Surgeons and others caring for children with craniosynostosis should be aware of the normal bimodal pattern of temperature spikes in the first 48 hours after corrective skull surgery, according to the researchers. Other patterns, especially if more complex, should lead to further evaluation for infection or other complications.

Source: Lippincott Williams & Wilkins